Recurrent sclerema in a young infant presenting with severe sepsis and severe pneumonia: an uncommon but extremely life-threatening condition.

A one month and twenty-five days old baby girl with problems of acute watery diarrhoea, severe dehydration, severe malnutrition, and reduced activity was admitted to the gastrointestinal unit of Dhaka Hospital of icddr,b. The differentials included dehydration, dyselectrolytaemia and severe sepsis. She was treated following the protocolized management guidelines of the hospital. However, within the next 24 hours, the patient deteriorated with additional problems of severe sepsis, severe pneumonia, hypoxaemia, ileus, and sclerema. She was transferred to the Intensive Care Unit (ICU). In the ICU, she was managed with oxygen supplementation, intravenous antibiotics, intravenous fluid, including a number of blood transfusions, vitamins, minerals, and diet. One month prior to this admission, she had been admitted to the ICU also with sclerema, septic shock, and urinary tract infection due to Escherichia coli and was discharged after full recovery. On both the occasions, she required repeated blood transfusions and aggressive antibiotic therapy in addition to appropriate fluid therapy and oxygen supplementation. She fully recovered from severe sepsis, severe malnutrition, ileus, sclerema, and pneumonia, both clinically and radiologically and was discharged two weeks after admission. Consecutive episodes of sclerema, resulting in two successive hospitalizations in a severely-malnourished young septic infant, have never been reported. However, this was managed successfully with blood transfusion, broad-spectrum antibiotics, and correction of electrolyte imbalance.

Risk factors for sclerema neonatorum in preterm neonates in Bangladesh.

BACKGROUND: This study presents a retrospective analysis of risk factors for sclerema neonatorum in preterm neonates in Bangladesh. METHODS: Preterm neonates admitted to Dhaka Shishu Hospital in Bangladesh were enrolled in a clinical trial to evaluate the effects of topical treatment with skin barrier-enhancing emollients on prevention of sepsis and mortality. Four hundred ninety-seven neonates were enrolled in the study and 51 (10.3%) developed sclerema neonatorum. We explored risk factors for sclerema neonatorum by comparing patients with and without sclerema neonatorum. Diagnosis of sclerema neonatorum was based on the presence of uniform hardening of skin and subcutaneous tissues to the extent that the skin could not be pitted nor picked up and pinched into a fold. Cultures of blood and cerebrospinal fluid were obtained in all neonates with clinical suspicion of sepsis. RESULTS: In multivariate analysis, lower maternal education (OR: 1.94; 95% CI: 1.02-3.69; P = 0.043), and signs of jaundice (OR: 2.82; 95% CI: 1.19-6.69; P = 0.018) and poor feeding (OR: 4.71; 95% CI: 1.02-21.74; P = 0.047) on admission were risk factors for developing sclerema neonatorum. The incidence rate ratio of sepsis in neonates who developed sclerema neonatorum was 1.81 (95% CI: 1.16-2.73; P = 0.004), primarily due to Gram-negative pathogens, and risk of death in infants with sclerema neonatorum was 46.5-fold higher (P < 0.001, 95% CI: 6.37-339.81) than for those without sclerema neonatorum. CONCLUSIONS: Sclerema neonatorum was a relatively common, grave condition in this setting, heralded by poor feeding, jaundice, and bacteremia, and signaling the need for prompt antibiotic treatment.

Predictors of mortality in infants with sclerema presenting to the Centre for Diarrhoeal Disease, Dhaka.

BACKGROUND: Sclerema is an uncommon, life-threatening condition, usually of newborns, with a case-fatality rate ranging from 50 to 100%. Very little is known about factors influencing outcome. AIM: To identify clinical and biochemical predictors associated with fatal outcome of sclerema in infants with diarrhoea. METHODS: Thirty infants with sclerema admitted to the Special Care Unit of the Dhaka Hospital of ICDDR,B with diarrhoea from May 2005 to end April 2006 were studied prospectively. Nine infants who died (30%) were considered to be cases while the 21 who survived constituted the comparison group. Hypothermia, severe malnutrition, septic shock, serum ammonia and CRP levels were considered to be predictors of death. Differences in proportions were compared by the chi(2) test and mean differences were compared using Student's t-test or the Mann-Whitney test, as appropriate. RESULTS: The mean age of the 30 infants was 2.1 months (range 12 d to 8 m). Fatal cases were more likely than survivors to be associated with severe underweight, a positive blood culture and higher serum ammonia and serum CRP levels. After adjusting for possible confounders in logistic regression analysis, the likelihood of death was higher in infants admitted with septic shock or who developed it soon after admission (OR 17.96, 95% CI 1.5-0220.4, p=0.024). CONCLUSIONS: Sclerema is associated with a high fatality rate and scleremic infants with diarrhoea who present with septic shock are at a greater risk of death.

Bebé colodión: manejo y proceso diagnóstico / Collodion baby: management and diagnosis

El «bebé colodión» es una situación clínica poco frecuente que se presenta en el neonato y que es compartida por varias enfermedades y síndromes. La alteración de la barrera epidérmica hace que el manejo y soporte del neonato sean fundamentales en los primeros días de vida, para luego llegar a un diagnóstico adecuado. Presentamos un nuevo caso de esta entidad en la que la colaboración del neonatólogo y el dermatólogo es fundamental

Collodion baby is a rare clinical condition affecting the newborn that is common to several diseases and syndromes. The alteration of the skin barrier makes management and care key factors in the first few days of life, after which the efforts are focused on reaching an accurate diagnosis. We present a new case of this clinical entity in which collaboration between the neonatologist and the dermatologist is essential

Exchange transfusion in septic neonates with sclerema: effect on immunoglobulin and complement levels.

OBJECTIVE: To study the effect of exchange transfusion (ET) on the levels of immunoglobulins (Ig) and C3 in neonatal sepsis with sclerema. DESIGN: Randomized controlled trial in a referral neonatal unit of a teaching hospital. SUBJECTS: Consecutive culture positive septic neonates with sclerema were enrolled and were randomized to undergo ET (study group, n = 20) or no ET (controls, n = 20). RESULTS: Mortality was 50% in the study group and 95% in controls. Gram negative organisms accounted for 85% in study group and 90% in controls. IgG, IgA and IgM levels rose significantly while C3 levels did not show significant rise 12-24 hours after ET. Ig and C3 levels did not change significantly in the controls. CONCLUSION: ET with fresh whole blood in septicemic newborns with sclerema improves survival, particularly in the more premature group and significantly enhances, IgG, IgA and IgM levels.

Epidemiological study on hypothermia in newborns.

A study on the incidence and high risk factors of hypothermia in the newborn was carried out in a period of four months (Nov. 1988-Feb. 1989) in 6 counties of 3 provinces. Totally 14,809 newborns were studied, in whom 100 cases were found to be ill with sclerema, having an incidence of 6.7%. Gestational age, body weight, low room temperature, improper methods of thermopreservation during delivery and asphyxia are determined as the most probable high risk factors, in which gestational age and body weight are negatively correlated with the contraction of the disease. It suggests that the prevention of prematurity and asphyxia, and promotion of thermopreservation during delivery should be helpful in preventing sclerema of newborns.

Sclerema neonatorum and subcutaneous fat necrosis of the newborn in the same infant.

Sclerema neonatorum (SN) is a rare disease of the newborn characterised by diffuse hardening of the subcutaneous adipose tissue. The condition is distinct from the localised violacious nodules observed in subcutaneous fat necrosis of the newborn (SCFN). Both conditions were diagnosed within the first 2 weeks of life in the same infant. SN and SCFN are unusual conditions in the newborn, the former usually associated with a severe clinical illness, following delivery or major surgery. The skin lesions of these two conditions are distinct and do not usually occur simultaneously. SN is associated with a poor prognosis.

Role of exchange transfusion in the treatment of severe septicemia.

Ten critically ill newborn infants presenting with documented septicemia were treated with antibiotics and supportive measures that included assisted ventilation, large blood transfusions and other volume expanders, sodium bicarbonate, and vasoactive drugs. Upon failure of the above treatment to improve the infants' rapidly deteriorating condition and the development of sclerema, exchange transfusions with fresh whole blood were performed and repeated up to four times. Seven of the ten infants showed immediate improvement and ultimately survived. IgM and IgA rose consistently with exchange transfusions. We postulate that these infants improved following exchange transfusion as the result of the removal of endotoxins, improvement of perfusion and of tissue oxygenation, decrease of hemorrhagic complications, and enhancement of the humoral and cellular inflammatory response. The development of sclerema in septicemic newborn infants continues to be an ominous sign despite the use of antibiotics and supportive measures. Our data suggest that exchange transfusions decrease the mortality of this group of critically ill infants.

Exchange transfusion in severe neonatal infection with sclerema.

Two critically ill newborn babies with severe infection associated with sclerema were successfully treated with appropriate antibiotics and repeated exchange transfusions.